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Early identification of patients likely to deteriorate post-hepatic portoenterostomy for biliary atresia (BA) would be beneficial. alpha-Glutathione-s-transferase (alpha-GST) is a serologic marker of reactive hepatocellular damage because of its low molecular weight, uniform hepatic distribution, high cytosol concentration, and short half-life. We evaluated whether serum alpha-GST in post-surgical BA patients correlates with liver function (LF) and investigated its potential as a medium- to long-term marker of prognosis. Postoperative BA patients (n = 30; mean age: 11.8 +/- 3.7 years) were divided into three groups based on average LF over the 3 months prior to this study. Group I (n = 8) were jaundice-free and had normal LF. Group II (n = 12) had moderate liver dysfunction, and group III (n = 10) had severe liver dysfunction. Serum alpha-GST was determined using a specific ELISA. Tissue alpha-GST was determined immunohistochemically, using liver needle-biopsy specimens. Bile lakes were found in 5 group II patients and 5 group III patients. Serum alpha-GST was significantly higher in group II (20.7 +/- 8.4 ng/ml) than in groups I (4.7 +/- 1.3 ng/ml) and III (8.0 +/- 1.2 ng/ml) (P0.0001) and was highest in group II subjects with bile lakes. In control liver specimens alpha-GST distribution was weak but uniform throughout normal liver lobule hepatocytes. In group II there was strong staining in centrilobular hepatocytes, and in group III alpha-GST was only found in regenerative nodules. We conclude that alpha-GST may be a more sensitive indicator of hepatocellular damage in BA because its distribution is correlated to the proportion of functioning liver tissue present. This is the first report of this relationship, which has great implications for group II subjects because a sudden shift in concentration of alpha-GST may be a better predictor of impending hepatic dysfunction than conventional LF tests.

Background and Purpose: Electrosurgery is the predominant form of energy used in open and laparoscopic surgery despite its well-recognized risks. In 1992, an ultrasonically activated scalpel—Harmonic Scalpel (laparoscopic coagulating shears; LCS)—a device that achieves effective coagulation and safe cutting of tissue, avoiding the risks of electrosurgery, was introduced. The aim of this study was to evaluate the effectiveness of the LCSs in pediatric laparoscopic surgery. Patients and Methods: To test the advantages of the LCS in pediatric laparoscopic surgery, we selected six different laparoscopic procedures: primary pull-through for Hirschsprung's disease, Toupet fundoplication, nephrectomy, splenectomy, salpingooophorectomy, and appendectomy. Patients were assigned to two groups prospectively and randomly for each procedure: Lap-LCS (using LCS) or Lap-electro (using conventional electrosurgery). These two groups were compared according to the various laparoscopic procedures with regard to ope...

This study compared laparoscopy-assisted stoma closure (Lap) with conventional closure (Co) to assess loss of intestine. Ileostomies (loop L; single S) were performed 5 cm proximal to the ileocecal junction through a right lower quadrant incision in forty 11-week-old Lewis rats (L = 20, S = 20). Stoma closure was performed 60 days later using laparoscopy (Lap) or conventional closure (Co) in 10 rats each, to give 4 groups, Lap-L, Lap-S, Co-L, and Co-S. End-to-end anastomosis was performed through the stoma site in all rats. Bowel resected from the skin to the anastomosis was termed resected unusable bowel (RUB) and measured blindly. Laparotomy was performed 30 days later to assess the status of the anastomosis and complications. Average RUB with Lap was significantly shorter; Lap-L (17.8 mm) versus Co-L (23.8 mm), P = 0.002, and Lap-S (10.6 mm) versus Co-S (13.8 mm), P = 0.001. During Co, accidental full-thickness injury to underlying bowel during stoma take-down occurred in 3 Co-L and 2 Co-S rats. All Lap rats were uncomplicated. Average times taken until end of stoma take-down were 6.1 min for Lap-L (3.2 min for trocar insertion, 2.8 min for stoma take-down), 5.6 min for Lap-S (2.8 and 2.7 min), 6.3 min for Co-L (from first incision to stoma take-down), and 5.1 min for Co-S (P = NS). At laparotomy there was no evidence of complications such as wound infection, incisional hernia or anastomotic stenosis in any rat. Our results suggest that laparoscopy-assisted stoma closure is safe and quick, and results in less loss of intestine during stoma closure.

We encountered a case of pectus excavatum associated with accessory diaphragm that was successfully excised thoracoscopically during the Nuss procedure. To the best of our knowledge, this is the first time for this condition to be reported in the literature.

Hepatic fibrosis can progress in biliary atresia (BA) and is associated with capillarization of hepatic sinusoids. The significance of serum hyaluronic acid (HA) as a noninvasive indicator of histological sinusoidal endothelial cell (SEC) damage and hepatic fibrosis in BA, is investigated.A total of 28 postoperative BA patients (mean age, 11.0+/-3.7 years) and 20 normal controls (mean age, 10.5+/-2.8 years) were studied. BA patients were divided into group I, good liver function (n = 8); group II, moderate liver dysfunction (n = 10); and group III, severe liver dysfunction (n = 10). Serum HA was determined using a one-step sandwich enzyme immunoassay, and liver histological damage was confirmed immunohistochemically using an antibody against factor VIII-related antigen (FVIIIRAg), which is specific for detecting damaged SEC.Serum HA was significantly higher (P.0001) in group III (84.6+/-36.5 ng/mL) than in group I (15.9+/-6.9 ng/mL) or group 11 (28.7+/-10.7 ng/mL). Although immunoreactive products of FVIIIRAg were abundant in group III, they were not detected in SEC from group II.Serum HA may be of value for monitoring postoperative BA patients as a noninvasive indicator of SEC damage and progressive hepatic fibrosis.

Background/Purpose : The aim of this study was to review complications after sigmoidocolocystoplasty (SCP) performed at a single institution from 1984 to 1997. Methods : The medical records of 100 patients who underwent SCP were reviewed retrospectively. Results : Mean age at SCP was 10.8 years. Urinary control was improved in 75 cases and unchanged in 25. Post-SCP complications included death, abdominal wound infection or dehiscence, adhesive bowel obstruction, vesical calculi, vesicocolonic anastomosis stenosis, metabolic acidosis, and transient renal hypertension. Fifty-one patients underwent ureteric re-implantation (URI) at the time of, or before, SCP, and 7 had recurrence of VUR post-URI (spontaneous regression in 6); 3 patients had new onset of contralateral VUR post-URI (spontaneous regression in 2). Transient pleural effusion was seen after reinsertion of ventriculo-pleural shunts to ventriculo-thoracic in 12 cases, but there was no incidence of infection. Squamous metaplasia of the bladder mucosa was identified in 5 patients on routine mucosal biopsy results but resolved in all cases after regular bladder irrigation was commenced. Preoperative constipation or fecal incontinence was better managed after sigmoidectomy in approximately one third of cases (38%). Conclusions : SCP with or without URI can improve the quality of life of patients with neurogenic or small-capacity bladder, but it can be associated with long-term complications. Regular bladder irrigation is recommended to maintain bladder mucosa integrity.

Objective To examine whether Lapprotector™, a protective film and ring device for protectingwounds, can prevent wound infection after open appendectomy. Methods We performed open appendectomy on 64 patients between 2004 and 2006. In September 2005, we started using Lapprotector™ to protect the site of incision (McBurney's point). Patients were dividedinto two groups as follows: Lapp(−), n =32; Lapp(+), n =32. Patient demographics were not statisticallydifferent and antibiotic protocols were identical. Results In the Lapp(−) group, the appendix was perforated in seven patients (21.9%) and not perfo-rated in 25 patients (78.1%). In the Lapp(+) group, the appendix was perforated in nine patients (28.1%) and not perforated in 23 patients (71.9%). For perforated cases, incisional wound infection was seen in three out of seven patients (42.9%) in the Lapp(−) group and in no patient (0%) in the Lapp(+) group, a significant difference ( p 2 test). For nonperforated cases, wound infection was seen in only one out of 25 patients (4.0%) in the Lapp(−) group. Conclusion We recommend using Lapprotector™ to prevent incisional wound infection in patientsrequiring open appendectomy, especially in cases where the appendix is perforated.

A 14-year-old adolescent girl with superior mesenteric artery syndrome was referred to us after failure of conservative management. Anterior transposition of the duodenum was performed and appears to be safe for permanently circumventing the duodenal obstruction seen in superior mesenteric artery syndrome, even in a pediatric population.

Complete covered cloacal exstrophy (CCCE) is extremely rare. The anatomy of CCCE is complex and often unique, and each case must be treated individually. We present the case of a 5-year-old girl with CCCE whom we treated successfully with great improvement in her quality of life.

Recessive dystrophic epidermolysis bullosa is a rare disorder characterized by extreme vulnerability of the squamous epithelium and mucous membranes. Minor trauma such as is caused by swallowing solid food is followed by blistering and scarring. Stricture formation at the pharyngoesophageal junction (C6 or C7) is the severest complication of this disease.We evaluate the effectiveness of Microvasive Rigiflex balloon dilatation and extensive nutritional support as a primary treatment for this condition. Nine of 21 recessive dystrophic epidermolysis bullosa patients developed esophageal strictures at the level of the pharyngo-esophageal junction (C6). We treated them with intensive nutritional therapy followed by balloon dilatation, which produces longitudinal pressure and provides prompt relief from esophageal stricture. Eleven balloon dilatations have been performed in 9 patients.All patients had poor physical development and were severely malnourished; extensive nutritional support was required before treatment could begin. Balloon dilatation was performed once in seven patients and twice in two patients. No recurrent stricture formation was observed after balloon dilatation.Intensive nutritional support followed by balloon dilatation is the first choice of treatment for esophageal strictures complicating recessive epidermolysis bullosa. By following this regime, invasive surgery can be avoided.

In the gut, C-KIT is important for immune system homeostasis, and C-KIT+ cells are known to increase during inflammation. Recently the authors identified that spontaneous intestinal mucosal erosion develops in C-KIT-depleted W/Wv mice after day 14 of life at a high frequency, whereas genotypically normal litter mates do not. The authors hypothesized that a lack of C-KIT may be implicated in the development of necrotizing enterocolitis (NEC).Bowel specimens were taken during surgery or postmortem from nine cases of NEC (mean gestational age, 32.0 weeks), six age-matched cases of enteritis, and 10 age-matched controls. Specimens were formalin fixed, paraffin embedded, and labeled with antibody to C-KIT. The number of C-KIT+ cells from five random fields per specimen were compared under light microscopy (200x). Results were expressed as the mean +/- SD and compared using the analysis of variance (ANOVA) test.In enteritis, the number of C-KIT+ cells in the lamina propria and submucosa was significantly higher than in controls (P.01) indicative of their involvement in inflammation. However, in NEC, the number of C-KIT+ cells in the lamina propria and submucosa was significantly lower than in controls (P.05) despite histological evidence of inflammation.A lack of C-KIT+ cells may exert a causal influence on the development of NEC.

To study the surgical treatment and outcome of hydronephrosis secondary to pelviureteric junction obstruction (PUJS) that is so massive that the renal pelvis crosses over the vertebral column [mega-hydronephrosis (MH)]. Of the 40 cases of PUJS we treated over the past 5 years, 6 cases had MH secondary to PUJS (MH-PUJS) in 6 renal units and were reviewed. Incidence of MH was 15.0%. All had Anderson-Hynes dismembered pyeloplasty to treat obstructive renal pattern on technetium-99m diethylenetriaminepentaacetic acid (DTPA) scans in five and gait disturbance due to MH in one. The contralateral kidney was normal in five and dysplastic in one. Three had nephrostomy before pyeloplasty. Preoperative DTPA scans showed good renal function in four and poor in two. Plication of the entire dilated, lax renal parenchyma ("nephroplication") was performed in three to decrease the intra-renal pelvic cavity. Prenatal diagnosis was performed in three cases. Mean age at pyeloplasty was 3.5 years and mean duration of follow-up was 1.5 years. Postoperatively, two of the three who did not have nephroplication had prolonged but not obstructed renal pattern on DTPA scans, and the other case who did not have nephroplication had nephrectomy for non-functioning kidney caused by recurrent pyelonephritis 2 years after pyeloplasty. In the three who had nephroplication, DTPA scans showed acceptable to relatively good passage in all cases. The surgical treatment of MH-PUJS is challenging with no fixed guidelines. However, nephroplication may be useful during pyeloplasty to decrease the size of MH-PUJS.

To evaluate the clinical benefits and problems associated with pediatric endosurgery, we analyzed our own experience of laparoscopy in children. From 1992 to 1996, 88 children underwent endosurgery at our hospital. Ages ranged from 2 days to 16 years; 23 were neonates. To confirm the safety of carbon dioxide (CO2) gas insufflation in children, especially in neonates, we monitored the end tidal CO2 level, oxygen (O2) saturation, blood pressure, and body temperature during endosurgery. Interleukin-6 (IL-6) and C-reactive protein (CRP) were measured serially to estimate the degree of surgical stress encountered during endosurgery and were compared with results obtained from patients who had undergone open surgery. CO2 gas insufflation was safely carried out in most cases at a speed of 1.0 L/min to an intra-abdominal pressure of 8 to 10 mmHg. However, hypothermia due to rapid insufflation of cool CO2 gas was encountered in 4 neonates insuffflated under these conditions. We have not encountered this c...

Traditionally, choledochal cyst (CC) is classified into five types according to Todani's classification. Of 356 cases of CC treated at our institute since 1965, we experienced four cases of mixed type I and II choledochal cyst (MTCC). The incidence of MTCC was 1.1%. All four patients had a fusiform common bile duct (CBD) with a diverticulum originating from the mid-portion of the CBD. The cystic duct drained into the right side of the diverticulum. All had pancreaticobiliary malunion (PBMU). All presented with recurrent abdominal pain. MTCC was suggested on ultrasonography or computerized tomography and confirmed by endoscopic retrograde cholangiopancreatography (three cases) or magnetic resonance cholangiopancreatography (one case). Age at surgery ranged from 3 to 12 years. All had hepaticoenterostomy, and total excision of the CC/diverticulum/gallbladder. There were no complications and all cases are currently well. In all cases, histopathology showed that the wall of the diverticulum was identical to the CBD. Although the incidence of MTCC is very low, pediatric surgeons should be aware of it as a new subtype of CC. Based on our experiences and a review of the literature, we suggest that its etiology does not affect treatment because the surgical management of choice is the same as for type I CC.

Rectal atresia is a rare condition in which the anus and sphincter muscles are normally developed, with usually no fistulous communication with the urinary tract. An unusual case of rectal atresia associated with recto-bulbar urethral fistula treated by a combination of colo-anal anastomosis and mucosal proctectomy via a posterior sagittal approach is reported for the first time.

Although thoracoscopic lobectomy for severe bronchiectasis has been reported in children, this is the fi rst report of double lobectomy of the right middle and lower lobes performed using the video-assisted thoracoscopy in a 9-year-old girl. The post-operative course was uneventful and she is currently well after 18 months’ follow-up.Key words: Bronchiectasis, children, double lobectomy, video-assisted thoracoscopy

We report four cases of long-gap esophageal atresia (LGEA) treated with thoracoscopic esophagoesophagostomy (TEE) after sequential extrathoracic esophageal elongation (SEEE: Kimura’s technique). All initially had gastrostomy, then SEEE. The proximal end of the esophagus was introduced into the apex of the thorax under direct vision during TEE. Mean birth weight was 1.9 kg. Mean gestational age was 35.0 weeks (range 30–39); mean age at initial esophagostomy was 43.2 days (range 15–110); SEEE was performed for a mean of 3 times (range 2–4) at mean intervals of 5.3 months (range 2–10), with the upper esophageal segment lengthened by a mean of 2.1 cm each time (range 1.1–3.5). Mean age and weight at TEE were 22.3 months and 9.8 kg, respectively. Gap was initially 4.5 vertebrae (range 4–5). Mean operating time was 9.6 h. TEE was successful in 3; 1 required thoracotomy, then re-anastomosis after 11 months of anastomosis leakage; 2 cases with post-TEE anastomosis leakage were treated conservatively. Postoperatively, all cases required fundoplication and esophageal dilatation (2, 3, 5, 8 times, respectively). At mean follow-up of 3.6 years, 2 eat normally and 2 eat minced food. TEE after SEEE appears to be a feasible option for treating LGEA.

To compare open repair (OR) with thoracoscopic repair (TR) for congenital diaphragmatic hernia (CDH) in neonates.Twenty-four neonatal CDH cases diagnosed prenatally or within 6 h of birth at our institute from 2002 to 2012 with mild pulmonary hypertension managed without inhaled nitric oxide, were studied. OR was routine until 2006 (n = 14; L:R = 12:2) and TR became routine in 2007 (n = 10; L:R = 10:0). All subjects had identical management.Gestational age at birth: OR 37.4 ± 1.6 vs. TR: 38.3 ± 1.1 weeks (p = 0.10); birth weight 2,636 ± 490 vs. 2,887 ± 429 grams (p = 0.20); preoperative A-aDO(2): 308 ± 200 vs. 331 ± 195 mmHg (p = 0.79); and operating time (min): 161 ± 42 vs. 194 ± 76 (p = 0.27). In TR, intraoperative cardiopulmonary status was stable, intraoperative hemorrhage was significantly less (4.8 ± 6.0 vs. 1.1 ± 0.1 grams; p = 0.038), wound cosmesis was excellent without surgical site infections, while, maximum postoperative CRP (2.0 ± 1.5 vs. 1.5 ± 1.1 mg/dL; p = 0.30), commencement of enteral feeding (6.9 ± 2.8 vs. 5.6 ± 2.8 days; p = 0.27), and length of postoperative hospitalization (36 ± 22 vs. 34 ± 12 days; p = 0.66) were improved, but not significantly. One OR case required surgery for intestinal obstruction.Thoracoscopic repair appears to be as effective as OR for treating selected cases of CDH in neonates, with excellent wound cosmesis.

Retroperitoneoscopic nephrectomy (R-neph) is still not generally favored by pediatric surgeons for various reasons, including lack of experience of retroperitoneal anatomy compared with pediatric urologists, concern about long operative times, and related complications.We compared nephrectomies/heminephrectomies planned, performed, and managed by five senior pediatric surgical trainees (SPST) under the supervision of a board-certified pediatric surgeon (BCPS) (A.Y.) using four-trocar retroperitoneoscopy (R-neph) (n=11) with conventional open nephrectomy and heminephrectomy (O-neph) (n=20) performed by 4 BCPS.O-neph comprised 14 nephrectomies and 6 upper pole nephrectomies; R-neph comprised 9 total nephrectomies and 2 upper pole nephrectomies. Mean age and mean weight at nephrectomy were not statistically different. Mean operating time (MOT) was 137 (range, 85-290) minutes in O-neph versus 197 (116-341) minutes in R-neph. MOT for the first 5 R-neph cases was 249 minutes versus 153 minutes for the last 6 cases. Mean blood loss was 17 (range, 1-55) mL in O-neph versus 10.3 (2-40) mL in R-neph. One R-neph case required conversion to O-neph. There were no transfusions and no intraoperative complications. Two partial heminephrectomy patients (one O-neph and one R-neph) developed transient urinoma postoperatively that resolved conservatively. Mean duration of postoperative bed rest was 1.0 day in O-neph versus 0.6 days in R-neph. Differences in mean postoperative fentanyl requirement (O-neph, 21.5 [10-40] μg/kg; R-neph, 4.1 [0-20] μg/kg) and duration of nonsteroidal anti-inflammatory suppository usage (O-neph, 2.3 [0-5] days; R-neph, 0.9 [0-2] days) were significant (both P.05). Full oral feeding was resumed after a mean of 1.6 (1-2) days in O neph and 1.2 (1-2) days in R-neph.R-neph was safely performed by SPST, and results were comparable to those with O-neph performed by BCPS.

The effectiveness of pull-through for Hirschsprung's disease is dependent on accurate identification of normoganglionic bowel in intraoperative biopsy specimens. We report 2 cases of patchy innervation of pull-through bowel in children with Hirschsprung's disease only identified by circumferential biopsying. Case 1 was an 8-month-old boy. During laparoscopy-assisted transanal endorectal pull-through, extra biopsies of bowel were taken circumferentially, 2 cm proximal to the level of normoganglionosis confirmed by laparoscopic colon biopsies. Aganglionosis was found at 3 o'clock, suggesting that bowel innervation at this level was patchy. Circumferential biopsies were performed a further 2cm proximally, and all sites were normoganglionic. This level was used for pull-through with excellent outcome. Case 2 was a 27-day-old boy. Similarly, extra biopsies were taken circumferentially, 2cm proximal to the level of "normoganglionosis" as indicated by conventional biopsying. Normoganglionosis was found only at 3 o'clock, while all other sites were hypoganglionic. A further series of circumferential biopsies was performed 2 cm proximally and hypoganglionosis was still identified, but only at 6 o'clock. Circumferential biopsies were repeated another 2cm proximally, and all sites were normoganglionic. We recommend circumferential biopsies be performed routinely to prevent bowel with patchy innervation from being used for pull-through and possibly causing postoperative bowel dysmotility in a subgroup of Hirschsprung's disease patients.

It is known that the etiology of congenital biliary dilatation (CBD) is closely associated with pancreaticobiliary maljunction (PBMJ). Treatment of CBD today is primary excision of the cyst followed by hepaticoenterostomy. However, PBMJ without dilatation of the biliary tract has recently been reported and its treatment is still controversial. In adults, simple cholecystectomy without biliary reconstruction is often performed, because it usually presents as an anomaly of the gallbladder. Over 30 years, we have encountered 8 patients with PBMJ without dilatation of the common bile duct in 180 pediatric cases of CBD. The presenting symptoms of these patients were those of pancreatitis i.e. abdominal pain associated with elevation of serum amylase levels. Five cases presented with jaundice or a history of pale colored stools. Endoscopic retrograde cholangiopancreatography was performed to confirm the diagnosis. Five (62.5%) of these 8 patients were found to have dilatation of the common channel, which was seen in 50 (29%) of the 172 other cases with biliary tract dilatation. Three cases (37.5%) had proven protein plugs or debris at the level of the common channel, and this was observed in 28 (16%) of the 172 other cases. It is strongly suggested that the manifestation of clinical symptoms in these patients results from stasis or obstruction at the level of the common channel. In children who present with recurrent pancreatitis, PBMJ must be suspected even if the common bile duct appears to be normal. It is difficult to resolve these anomalies without operative repair. The authors consider that radical treatment of PBMJ is required in these children to prevent serious long-term complications.

There has been no report describing suspected prenatal pyriform sinus (PS) cyst and fistula. We report a case suspected by prenatal ultrasonography and fetal MRI. A large cystic mass was found in the left neck of the fetus. After the baby was born, preoperative laryngoscopic catheterization of the fistula tract was used to confirm the diagnosis and greatly facilitated the identification and excision of the PS cyst and fistula.

We investigate the incidence, treatment, and implications of intrahepatic bile duct (IHBD) dilatation debris in 42 patients with choledochal cyst treated over a recent 8-year period. Mean age at cyst excision was 6.9 years, and mean follow-up period was 5.4 years. Intraoperative endoscopy (IOE) was performed at the time of cyst excision using a pediatric cystoscope, which identified IHBD debris in 11/42 (26.2%), and massive debris in 4/42 (9.5%). In all cases, IHBD debris was successfully washed out with normal saline through the cystoscope. There was no postoperative IHBD stone formation or cholangitis except for one case of cholangitis in a patient undergoing chemotherapy for acute myelocytic leukemia. Debris left in the IHBD during cyst excision is probably one of the primary causes of postoperative IHBD stone formation and can be prevented by washing during IOE.

Purpose: The aim of this study was to review the outcome of a protocol the authors proposed for preventing Roux-en-Y hepatico-jejunostomy (RYHJ) related complications. Methods: In 1986 the authors adopted the following protocol for improving RYHJ to minimize risks for RY-related complications: (1) end-to-end anastomosis if possible; (2) if end-to-side anastomosis is unavoidable, the common hepatic duct should be anastomosed as close as possible to the closed end of the blind pouch (BP); (3) careful selection of the vascular supply to the RY jejunal limb; (4) length of the RY jejunal limb should not be predetermined, but individualized; (5) approximate the native jejunum to the RY limb. The authors reviewed 92 children who had surgery for CC (mean age at surgery, 4.1 years) at our institution between 1986 and 2002 to assess the effectiveness of their recommendations. Results: After the adoption of the authors' recommendations for RYHJ, they have experienced no RYHJ-related complications after a mean follow-up period of 7.6 years (range, 4 months to 16 years). Conclusions: RYHJ in children is different from that in adults, because the RY limb or BP can grow and elongate as the child grows. The authors' recommendations appear to have successfully prevented RY-related complications up to the time of this review, and they highly recommend their use.

The authors present 3 cases of proximal hypospadias associated with bilateral undescended testes (UDT) in which the gubernaculum was used to reinforce the neourethra to prevent urethrocutaneous fistula. This is the first report of the gubernaculum being used to reinforce the neourethra. The technique is simple and should be applicable to any hypospadias patient with concomitant UDT, especially in severe hypospadias. Good outcome is likely because of the excellent blood supply of the gubernaculum.

Lymphangioma of the tongue is relatively rare and may cause facial structural deformity. Using a combination of a V-shaped and central resection, we successfully treated a 6-year-old girl who had massive lymphangioma of the tongue. Postoperatively, her tongue was located completely within her mouth with good cosmetic results. Sensory and motor nerves to the tongue appeared to be intact. Her speech was also improved.

We assessed continence after scope-assisted anorectovaginoplasty (SARVP) for female anorectal malformation (FARM). Five FARM cases were assessed; cases 1 and 2: cloacal malformation; case 3: urogenital sinus, and rectovestibular fistula (RF); case 4: RF, absent vagina, and sacral anomaly; case 5: covered cloacal exstrophy. Treatment was SARVP in all cases, with perineal vaginoplasty (case 1), vagina pull-through (PT) similar to Georgeson’s colon PT (case 2), and the use of the native RF/cloaca channel as a vagina (cases 3–5). Continence was assessed pre and postoperatively. SARVP was performed in the lithotomy position without repositioning. Mean age at surgery was 3.2 (1.7–5.5) years. Current mean age: 8.8 years (range 7.5–12.2). Mean follow-up: 5.7 years. Preoperative continence: fecal: all had stomas; urinary: cases 1 and 2: continent; cases 3–5: incontinent. Postoperative continence: fecal: cases 1–3: continent; case 4: incontinent; case 5: awaiting stoma closure; urinary: cases 1 and 2: continent; cases 3 and 4: incontinent; case 5: continent (intermittent catheterization). Fetal continence evaluation questionnaire (CEQ) scores for cases 1–4 were 7.5, 9, 10, and 2 (maximum score 10, mean 7.1). Scope assistance improves visualization, thus pelvic sphincter dissection/division is minimized with less detrimental impact on postoperative continence.

We reviewed 24 consecutive cases of prenatally or immediately postnatally diagnosed left-sided congenital diaphragmatic hernia (CDH) to evaluate pulmonary artery (PA) size as an indication for thoracoscopic repair (TR). CDH repair is planned once echocardiography confirms improvement in pulmonary hypertension. TR is chosen if cardiopulmonary status is stable more than 10 min in the decubitus position in the neonatal intensive care unit (NICU) under conventional mechanical or high frequency oscillatory ventilation (HFOV) with/without nitric oxide (NO) and the patient appears likely to tolerate manual ventilation during transfer to the operating room. Otherwise open repair (OR) is performed in NICU. Proximal right PA (RPA) and left PA (LPA) diameters measured at birth were assessed with respect to the type of repair. 10/24 had TR and 14/24 had OR. TR cases had significantly larger RPA/LPA diameters (3.52 ± 0.23 vs. 3.10 ± 0.56 mm, p

Minimally invasive surgery in children has evolved to the extent that complex procedures can be performed with safety and outcome comparable with open surgery, with the advantage of minimal scarring. Here we describe the latest laparoscopic techniques used by us at the Juntendo University Hospital, Japan, for treating biliary atresia and choledochal malformation, with presentation of our postoperative management and discussion of preliminary outcomes.

A 13-month-old boy accidentally ingested a 5 cm dressmaker’s pin. On presentation, the pin was in the duodenum and there was a right inguinal hernia. After 17 days, the pin failed to progress. At surgery, the sac contained appendix perforated by the pin. This is the fi rst case in the literature.

To determine if follow-up by consultant pediatric surgeons (CPS) affects morbidity due to incarceration (INC) in children with indirect inguinal hernia (IH). We educate parents so they can identify possible INC and advise them to attend our emergency department anytime for immediate review by on-call CPS. We reviewed 3,493 cases of IH by grouping them according to age at diagnosis: neonatal (G1; n = 96), 2–3 months (G2; n = 331), 4–6 months (G3; n = 118), 7–12 months (G4; n = 193), and over 12 months (G5; n = 2,755). Data per group were: mean gestational age (weeks): 32.1, 38.0, 36.4, 37.4, 38.7; mean birth weight (g): 1,645, 2,736, 2,471, 2,769, 2,930; mean age at elective hernia repair (HR) (months): 11.3, 4.9, 10.1, 12.9, 56.5; mean weight at elective HR (kg): 6.8, 6.4, 7.3, 9.1, 17.4; mean duration from diagnosis to elective HR (months): 10.9, 3.1, 6.3, 3.6, 3.0; mean follow-up: 6.7 years. Overall, INC occurred in 203/3,493 during follow-up. Incidence per group was: G1: 4/96, G2: 62/331, G3: 6/118, G4: 47/193, G5: 84/2,755. All INC were reduced manually without complications. HR complications occurred in 7/3,493 (0.2%). With CPS follow-up, INC can be managed without morbidity, allowing elective HR to be performed later with fewer complications.

Snodgrass tubularized incised plate urethroplasty (SUP) is versatile and has good cosmesis. However, postoperative meatal/neourethral stenosis (M/N-S) is common enough for some surgeons to add a dorsal inlay graft (DIG) harvested from the inner prepuce and sutured to cover the longitudinal midline incision of the urethral plate. This is the first formal assessment of the effectiveness of DIG for preventing M/N-S.We reviewed the medical records of 100 consecutive SUP cases performed by a single surgeon between 2003 and 2010 comparing SUP + DIG (S + D group, n = 50) with SUP - DIG (S - D group, n = 50). Mean follow-up was 3.6 years. Data were analyzed statistically using the χ(2), 2-way ANOVA, and Mann-Whitney tests, with P.05 considered significant.Severity of hypospadias and type of SUP were similar. Mean age at SUP was 3.3 years in S + D and 3.6 years in S-D (P = NS). There were 4 complications in the S + D group: urethrocutaneous fistula (n = 3) and neourethral stenosis without diverticulum (n = 1). There were 15 complications in the S-D group : meatal stenosis (n = 2), neourethral stenosis with or without diverticulum (n = 6), urethrocutaneous fistula (n = 7) (P.01). M/N-S was significantly less in the S + D group (1 vs 8; P.05).We strongly recommend that DIG be performed routinely during SUP.

Liver herniation (LH) in congenital diaphragmatic hernia (CDH) may not be a reliable prognostic indicator. We measured pulmonary artery (PA) diameters in CDH + LH as an alternative.Of 41 consecutive cases of prenatally diagnosed left-sided CDH treated from 2002 to 2010, 19 had CDH + LH and 22 had CDH - LH. Ultrasonography and magnetic resonance imaging were used to assess LH and echocardiography to measure PA diameters during the third trimester (fetal; 32-34 weeks), at birth, and on day 2 of life.In CDH + LH survivors (9/19; 47%), fetal right PA (RPA) diameters were significantly larger than in nonsurvivors (2.58 ± 0.56 vs 1.82 ± 0.35 mm; P.01), but left PA (LPA) diameters were not (1.73 ± 0.38 vs 1.59 ± 0.22). In survivors, fetal RPA was greater than 2 mm in all but one case, and both PA diameters increased significantly by birth (RPA, 2.58 ± 0.56 vs 3.52 ± 0.54; LPA, 1.73 ± 0.38 vs 2.60 ± 0.40; both P.01). Final diameters at birth in survivors were at least 2.5 and 2.0 mm, respectively. In nonsurvivors, both PAs were significantly smaller (RPA, 3.52 ± 0.54 vs 2.04 ± 0.31; LPA, 2.60 ± 0.40 vs 1.68 ± 0.18; P.01), with no observed increase by birth. Survival in CDH - LH was 82% (18/22).PA diameter appears to be correlated with prognosis in infants with CDH + LH.

We tested our laparoscopy-assisted "open" ligation (LOL) technique on testicular vessels.We ligated the left testicular artery and vein (TAV) in 8-week-old male Wister rats using LOL (LOL group; n=10) or laparotomy (open group; n=10). In LOL, a 0-degree laparoscope was introduced through a 5-mm epigastric trocar. A 3-mm grasper was used to expose the left TAV. A lapa-her-closure (LHC) needle loaded with 3-0 SurgiPro was directly inserted into the left lower quadrant where the left TAV should be and advanced under the vessels, and the suture material was released leaving one end outside. The LHC was then withdrawn a little and advanced again over the vessels to grasp the end of the suture material just released to bring it outside. This was proximally repeated. The two ends of both sutures were conventionally tied outside, and the knot was passed through the insertion site and tightened around the vessels. In the open group, the left TAV were ligated using two 3-0 SurgiPro ties. In both groups, the right side was left intact. All rats were sacrificed 2 weeks postoperatively, and both testes were examined with hematoxylin and eosin.Treatment time was 5-7 minutes for LOL and 7-8 minutes for the open group. Postoperative recovery was uneventful. No adhesions were present between the ligated vessels and bowel in any rat. Histopathology of all left testes showed coagulative necrosis of germinal cells and seminiferous tubules; all right testes were normal.LOL appears to be as effective as open ligation and may find application for treating varicocele.

Laparoscopic Kasai portoenterostomy (LKPE) is generally regarded to have a poorer outcome than open Kasai portoenterostomy for the surgical treatment of uncorrectable biliary atresia. We will describe our LKPE as the only center using laparoscopy to perform Kasai portoenterostomy in Japan.For our LKPE, a 5-mm trocar is placed in the epigastrium in addition to conventional trocar placement. A Ligasure device is inserted through this additional trocar to seal portal vein branches at the porta hepatis draining into the caudate lobe, instead of hook diathermy that is used universally elsewhere because there is an unacceptably high risk for lateral thermal injury to microbile ducts. We minimize porta hepatic microbile duct injury during anastomosis between the Roux-en-Y jejunum and the liver parenchyma around the transected biliary remnant by not suturing where the original right and left bile ducts were present and making sutures deliberately shallow but deep enough to prevent leakage. Our anastomosis is more central to the porta hepatis, like the original Kasai, compared with "extensive lateral dissection" commonly performed elsewhere in Japan. The length of the Roux-en-Y jejunal limb should be individualized, not predetermined to be 30, 40, or 50 cm as is common practice; the jejuno-jejunostomy should fit naturally into the splenic flexure, otherwise the redundant limb may become tortuous as the patient grows, causing bile stasis and possible cholangitis.Our LKPE can be performed safely and successfully with encouraging outcome.

The aim of this study was to compare endoloops and endostaples for closing the stump of the appendix during laparoscopic appendectomy (LA) for uncomplicated appendicitis in children.All LA performed for appendicitis from 2005 to 2009 were analyzed prospectively. Cases of complicated appendicitis such as perforated appendicitis or intra-abdominal abscess were excluded, leaving 75 cases closed with loops and 81 cases closed with staples. Choice of technique was determined by the attending surgeon's preference. All patients were managed according to the same pre-, intra-, and postoperative protocols.There were no significant differences between mean age at surgery, gender ratio, preoperative mean white blood cell count and mean C-reactive protein, histopathologic findings, mean duration of surgery, and mean hospitalization. There were no intra-operative complications in either group, but one loop case required conversion to open surgery (P = NS). There were no significant differences in the incidences of intra-abdominal abscess, transient ileus, small bowel obstruction, and wound infection. The postoperative readmission rate for management of complications was 4.0% for loop cases and 2.5% for staple cases (P = NS). Overall, using staples for a standard LA (anesthesia and stump closure) was U.S. $405 more expensive than when loops were used.This is the first prospective study comparing endoloops with endostaples for closing the stump of the appendix during LA for uncomplicated appendicitis in children. Although stapling proved to be more expensive, the choice of technique should reflect the surgeon's experience and confidence to ensure that the patient has the safest possible LA.

We assessed ductal plate malformation (DPM) as a prognostic factor for mid-/long-term outcome in post-hepatoportoenterostomy (HPE) biliary atresia patients.Of 78 cases of HPE performed between 1989 and 2009, biopsy specimens were available for 43. Cytokeratin 19 was used to identify DPM and preoperative status, postoperative serum total bilirubin, time taken to become jaundice-free, duration of jaundice-free period, steroid usage, incidences of cholangitis, bile lakes, and varices, and requirement for reoperation and liver transplantation were compared.DPM was detected in 21/43 (48.8%) subjects [DPM-positive (DPM+)]. Differences were identified for maximum postoperative serum total bilirubin: DPM+ (12.28 ± 1.00 mg/dL) versus DPM-negative (DPM-) (9.67 ± 0.71 mg/dL) (P 0.05) and total steroid usage: DPM+ (98.3 ± 11.8 mg/kg) versus DPM- (83.4 ± 15.9 mg/kg) (P = NS). However, total steroid dose in jaundice-free cases was significantly higher in DPM+ [75.3 ± 10.0 mg/kg (n = 14) vs. 48.1 ± 6.4 mg/kg (n = 19); P 0.05)]. There were no differences for complications. Mean duration of follow-up was significantly shorter for DPM+ (60.9 ± 38.4 vs. 113.8 ± 56.9 months; P 0.05).Postoperative total bilirubin was higher in DPM+ cases, thus higher doses of steroids were required. However, DPM+ did not appear to influence the incidence of complications mid-/long-term.

Ectopic pancreas is of little clinical significance. We report a case of ectopic pancreas in a 2-year-old girl presenting as a heterogeneous solid submucosal tumor of the posterior wall of antrum proximal to the pylorus, treated by distal gastrectomy and Billroth I reconstruction. We reviewed 15 cases in the English literature.

We reviewed 26 consecutive cases of congenital diaphragmatic hernia (CDH) prospectively to establish selection criteria for successful thoracoscopic CDH repair (TR). Five preoperative deaths were excluded, leaving 21 subjects. TR was only considered once pulmonary hypertension (PH) improved on echocardiography, and if cardiopulmonary status was stable in the decubitus position in the neonatal intensive care unit (NICU) under conventional mechanical or high-frequency oscillatory ventilation (HFOV) with/without nitric oxide (NO) for at least 10 min as a marker for tolerating surgery and manual ventilation was possible for transfer to the operating room. All other patients had open repair (OR). 8/21 had TR and 13/21 had OR. There were significant differences between TR and OR for prenatal diagnosis (37.5 vs. 84.6%, p

A 16-year-old female with systemic lupus erythematosus was diagnosed with choledochal cyst. As high-dose steroids had been prescribed for CNS lupus, surgery was performed under steroid cover. Despite postoperative pneumonia and wound infection, she is well 2 years later. There is no similar report in the literature.

Reports on laparoscopic treatment (LT) of peritonitis secondary to perforated appendicitis (PA) in children often include localized peritonitis/appendiceal mass, without specifically referring to pan-peritonitis (PP). This study compared LT with open treatment (OT) in only those patients with PP secondary to PA.PP was defined as generalized abdominal rigidity on presentation with purulent material intraoperatively found throughout the entire abdominal cavity. Based on this definition, we identified 23 cases of PP secondary to PA from all our cases of appendicitis from 2004 to 2008 and prospectively collected data.Surgical intervention was LT in 12 cases and OT in 11 cases. Appendectomy was successful in all cases without intraoperative complications. Mean age at surgery, sex ratio, preoperative mean white blood cell count, and C-reactive protein were similar. Mean operative time was 119 minutes for LT and 107 minutes for OT (P = NS). Mean volume of saline used for peritoneal lavage was 2730 mL for LT and 2950 mL for OT (P = NS). Duration of analgesic usage was significantly shorter in LT (P = 0.01). Postoperative wound infections were significantly less in LT (P = 0.04: LT 0, OT 4/11). Adhesive bowel obstruction occurred in one LT case and three OT cases (P = NS); none required surgery. There were two cases of intraabdominal abscess in each group, all were conservatively treated. Time taken to become afebrile, for white blood cell count and C-reactive protein to normalize, for intravenous antibiotics to be ceased, and for oral feeding to be commenced were not significantly different. Mean hospitalization was significantly shorter for LT (P = 0.04).LT would appear to be superior for the treatment of PP secondary to appendicitis in children and would even seriously consider it as the procedure of choice.

Purpose The piebald lethal mouse with a deletion of endothelin-B receptor gene (EDNRB) is a model for Hirschsprung's disease (HD), whereas the SOX10 gene is vital for the development of intestinal neural crest–derived cells. Recently, we created a SOX10 transgenic mouse with intestinal neural crest–derived cells visible with enhanced green fluorescent protein (VENUS), that is, SOX10-VENUS + /EDNRB sl/sl to investigate intestinal innervation in HD. Methods SOX10-VENUS + /EDNRB sl/sl (n = 30) were compared with wild-type littermates as controls (EDNRB s/s , n=30). Mice were killed on days 3, 7, or 12 of age. The entire colorectum was excised, fixed with 4% paraformaldehyde, and examined using fluorescence microscopy alone without staining. Results In normoganglionic colorectum from controls, a grid network of nerve fibers/glial cells was visualized that connected smoothly with extrinsic nerve fibers running along the colorectal wall. In aganglionic colorectum from SOX10-VENUS + /EDNRB sl/sl mice, there was no grid network and more extrinsic nerve fibers than controls that invaded the colon wall becoming elongated with branching fibers. Normoganglionic colon from controls and SOX10-VENUS + /EDNRB sl/sl mice appeared the same. Innervation patterns did not change over time. Conclusion This is the first time for abnormal enteric innervation in aganglionic colon in a model for HD to be visualized without staining.

Aim The anorectal angle (AA) influences defecation after pull-through (PT) for imperforate anus (IA). We compared postoperative AA and continence after Georgeson's laparoscopy-assisted colon PT (GPT) and Pena's posterior sagittal anorectoplasty (PSARP) for high/intermediate-type IA. Methods We reviewed 33 high/intermediate-type IA cases (20 GPTs and 13 PSARPs) prospectively. All had colostomy initially as neonates. Anorectal angle was measured as the angle between the rectum and the anal canal on barium enema. A fecal continence evaluation questionnaire (FCEQ) consisting of 5 parameters (frequency of defecation, staining/soiling, perianal erosion, anal shape, and requirement for medication; maximum score=10) was evaluated in 28 cases (15 GPTs and 13 PSARPs) followed up for more than 3 years. Results Mean age at PT was similar (6.6 months for GPT and 6.3 months for PSARP; P = not significant). There was no significant difference in mean AA. The FCEQ scores for GPT were generally higher throughout the study and significantly better from 3 years postoperatively ( P Conclusions We are the first to confirm that effective AA similar to PSARP can be achieved after GPT, although FCEQ would suggest that GPT has less detrimental functional impact.

Anal canal duplication (ACD) is an extremely rare congenital intestinal anomaly. There are not many reports in the English literature, with just a few from each institution. The aim of this study was to describe the clinical characteristics, surgical treatment, and outcome of ACD in pediatric cases at a single institution. The medical records of ACD patients we treated from 1988 to 2009 were reviewed retrospectively. There were ten cases, all females. Nine were asymptomatic without any history of perianal infection, identified only incidentally at routine check-up although one case had severe periproctal abscess. Mean age at diagnosis was 6 months (range 0–47 months). Concurrent pathology present were meningocele and presacral mass in one, presacral mass in one, and renal hypoplasia in one. In all cases, an extraneous perianal orifice opening in the midline posterior to the native anus was present, but in one case it was only identified at surgery because it had closed secondary to severe inflammation preoperatively. All had preoperative radiologic investigations, such as magnetic resonance imaging (MRI), barium contrast studies, and fistulography. Mean age at surgery was 14 months (range 1–48 months). All underwent surgical removal of the anal canal duplication, eight through a perineal approach and two through a posterior sagittal approach, depending on the length of the duplicated anal canal and the presence of concurrent anomalies. Histopathology of the excised anal canals showed squamous epithelium in all cases. The postoperative course was uneventful in all cases and all are well without complications at mean follow-up of 9.1 years (range 5–19 years). This is the largest series of ACD at a single institution reported to date. For the clinician faced with an extraneous perianal orifice located posteriorly in the midline with respect to the native anus, ACD should be suspected. The treatment of choice in children is complete excision (perineal/posterior sagittal approach), even if asymptomatic. Morbidity is minimal, and outcome is excellent.

Pancreatoblastoma (PB) is extremely rare. A 11-year-old boy, who had excision of dilated common bile duct with hepaticoduodenostomy when 9 years old was referred to our institute for further management of abdominal pain and steatorrhea. Imaging studies showed a solid 4 cm tumor in the head of the pancreas and two lesions in the liver. Needle biopsies diagnosed PB with liver metastases. After five courses of chemotherapy, the primary tumor was completely resected with pancreaticoduodenectomy (PD) and Child’s pancreaticobiliary tract reconstruction. The liver metastases were resected. Postoperative recovery was uneventful. Here, we present this case and review the literature focusing on management of PB.

We report on technical modifications we developed for thoracoscopic esophagoesophagostomy in patients with esophageal atresia. They are: (1) placing stay sutures along the edges of the atretic esophagi and exteriorizing them through the thoracic wall to expose the luminal surfaces of the esophagi nicely and relieve tension on the anastomosis; (2) leaving 1/5 of the length of the distal and proximal ends of the atretic esophagi intact before placing the stay sutures, to avoid retraction of the mucosa into the lumen; (3) making the diameter of the proximal esophagus 1.5-2 times larger than the diameter of the distal esophagus to make the shape of the anastomosis more streamlined without notching.

Introduction We report a novel technique to measure the length of the rectourethral fistula (RUF) in male patients with high-/intermediate-type imperforate anus during laparoscopically assisted anorectal pull-through (LAARP) to prevent incomplete excision. Method During LAARP for RUF in 5 male patients (mean age, 4.6 months; prostatic in 3, bulbar in 2), the RUF was dissected carefully close to the urethra and opened; and a fine catheter with 10-mm calibrations was inserted by the laparoscopic surgeon until it was seen to emerge at or near the verumontanum by another surgeon performing cystoscopy. The laparoscopic surgeon then measured the distance from the point where dissection was ceased at the rectal end to the urethral orifice. The RUF was dissected free from the prostate for exactly this length, tied, and excised; and colon pull-through was performed to finish LAARP. Result Rectourethral fistulae ranged from 5 to 15 mm and were much longer than expected. All dissections were uncomplicated without any injury to the urethra, and postoperative courses were unremarkable. At mean follow-up of 11 months, urination is normal in all without evidence of residual fistula. Conclusion Knowing the exact length of the RUF facilitates safe and complete excision in an otherwise blind situation.

We report our experience of using a commercial wound protector and retractor system to allow spontaneous sutureless closure of the abdominal wall defect in gastroschisis.Following birth, eviscerated bowel is wrapped with polyethylene wrap, the umbilical cord is deliberately left long and kept moist, and the patient stabilized and transferred to the operating room. Then the Applied Alexis wound protector and retractor system (Applied Medical Resources Corp, USA) was used for reducing eviscerated bowel. Once all organs have been reduced, the wound protector and retractor system (WPAR) is removed and the abdominal wall defect covered with the umbilical cord without suturing. Tegaderm (3M Health Care, USA) is used as a film dressing and is removed once the umbilical cord becomes adhered.We have used this technique to treat seven neonates with gastroschisis between April 2006 and March 2009. In three, WPAR was used as a silo initially, and removed after 3-4 days, and in four, WPAR was used only until primary reduction was achieved and removed. The abdominal defect closed spontaneously in all cases with excellent cosmesis. There were no complications attributed to our technique. At the time of discharge, all patients had insignificant umbilical hernias which have all resolved spontaneously.The cosmetic appearance of the abdomen is improved using our technique compared with primary closure involving suturing and a conventional silo.

Hepatoblastoma originating in the caudate lobe is extremely rare, and resection is technically challenging. The aim of this study was to report our experience of complete resection of hepatoblastoma originating in the caudate lobe and review the literature. A 12-month-old boy was admitted for investigation of an upper abdominal mass. Imaging studies showed an irregularly lobulated 8-cm solid tumor in the left lobe of the liver. Serum alpha-fetoprotein was 67,700 ng/mL. This tumor was diagnosed as hepatoblastoma (PRETEXT II) and chemotherapy (3 courses of CDDP and ADR) was performed. After chemotherapy, the tumor had shrunk to a resectable size and its origin in the caudate lobe could be determined. Left hepatic lobectomy with en bloc resection of the caudate lobe was performed. Total blood loss was 10 mL, and the operation lasted 9 h. His postoperative course was uneventful. Preoperative chemotherapy facilitated complete resection by left hepatic lobectomy with total excision of the caudate lobe.

Laparoscopy-assisted stoma closure (LASC; n = 28) and conventional open closure (CSC; n = 24) were compared by assessing outcome in 52 consecutive cases of stoma closure (SC) in children.